Morbidity and mortality rate of the acute arterial ischemic stroke (AAIS) disease is high. The incidence of diagnosis in children have recently been increasing with wide use of imaging modalities. In this article, a pediatric case followed with diagnosis of AAIS is presented to draw attention to treatment strategies.15-year-old male patient was diagnosed as acute arterial ischemic stroke as a result of a sudden onset facial paralysis. In further evaluation for etiology of the patient, homozygous MTHFR A1298C and heterozygous PAI-1 4G/5G polymorphism was detected positively. Shortly after admission of the patient to the intensive care unit, cerebral edema was developed and mechanical ventilatory support and antiedema treatment was started. Although intensive antiedema treatment, uncal and tonsillar herniation developed and in the 19th day of hospitalization, he died the result of a sudden cardiac arrest. Because of poor prognosis despite the treatment in pediatric patients with the sudden onset of neurological symptoms; AAIS should always be considered in mind and should not be too late in treatment.